Immunosuppressive Therapy for Progressive Ocular Cicatricial Pemphigoid

In a controlled, nonrandomized, longitudinal cohort study, we studied the efficacy of systemic immunosuppression in treatment of progressive cicatricial pemphigoid affecting the eyes. Twenty-six patients were studied; 18 received systemic immunosuppressive drugs, and eight received conventional therapy. Three patients who received cytotoxic agents withdrew from the study with intolerable gastrointestinal distress. Fourteen of the 15 patients who were immunosuppressed successfully for prolonged periods experienced a cessation of their episodic ocular inflammation and a halting of their progressive conjunctival cicatrization. They maintained vision at least as good as that present when disease activity was brought under control; in two patients vision improved. The one patient who was adequately immunosuppressed and successfully maintained in such a state for two years but who had progressive ocular surface pathology with eventual blindness had concomitant rheumatoid arthritis and severe sicca syndrome. The three patients who withdrew from immunosuppressive therapy and the eight concomitant controls all showed continued episodic conjunctival inflammation and conjunctival cicatrization with development of severe keratopathy and profound visual loss. Complications in this series included alopecia (100%), anemia (78%), gastrointestinal distress (22%), hemorrhagic cystitis (11.1%), and severe leukopenia (5.5%). These results support the notion that abnormal immunoregulatory mechanisms are involved in the progressive disease activity in cicatricial pemphigoid and that systemic immunosuppression may have an appropriate role in the treatment of this disease.